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Journal of Ultrasound Sep 2021Paragangliomas are a rare form of highly vascularized tumors that originate from paraganglia Baysal (J Med Genet 39: 617-622, 2002). In the head and neck PGL arise...
Paragangliomas are a rare form of highly vascularized tumors that originate from paraganglia Baysal (J Med Genet 39: 617-622, 2002). In the head and neck PGL arise primarily in four distinct areas: vagal, middle ear, and larynx and more frequently carotid bifurcation. Imaging evaluations include sonography, color Doppler, US-elastosonography and contrast-enhanced ultrasound (CEUS). Additionally, Computed Tomography, Magnetic Resonance Imaging (MRI) as well as digital subtraction angiography can be performed Stoeckli et al. (Laryngoscope 112: 143-146, 2002). We present herein a case of a rare bilateral carotid body tumor assessed with multiparametric ultrasound evaluation, including CEUS and US-elastography.
Topics: Angiography, Digital Subtraction; Carotid Body Tumor; Humans; Magnetic Resonance Imaging; Tomography, X-Ray Computed; Ultrasonography
PubMed: 33999368
DOI: 10.1007/s40477-021-00581-z -
Journal of Clinical and Diagnostic... Jan 2016We report a large vagal neurilemmoma in the poststyloid compartment of the parapharyngeal space. A 52-year-old man was referred to our hospital with a feeling of...
We report a large vagal neurilemmoma in the poststyloid compartment of the parapharyngeal space. A 52-year-old man was referred to our hospital with a feeling of discomfort in the left upper neck. Computed tomography showed a 30mm x 30mm x 40mm mass with inhomogeneous internal enhancement in the left carotid space. Magnetic resonance imaging revealed a 30mm × 30mm × 40mm heterogeneous mass in the area of the bifurcation of the common carotid artery. We gave a provisional diagnosis of neurilemmoma or vagal paraganglioma in the parapharyngeal space preoperatively based on the results of physical examination and imaging. We selected a transcervical-transmandibular approach. Under general anaesthesia, a tumour originating from the vagus nerve was completely extirpated while protecting the internal and external carotid arteries. Although mild postvagotomy dysphagia and hoarseness were seem for 6 months postoperatively, symptoms resolved and the patient showed a satisfactory course without recurrence after 10 years. Histological examination of the excised specimen showed antoni A and antoni B pattern. Positive immunoreactivity for S-100 protein was identified, but negative results were obtained for neuron-specific enolase, chromogranin and neurofilament. The tumour was diagnosed as neurilemmoma of the vagus nerve.
PubMed: 26894190
DOI: 10.7860/JCDR/2016/13789.7141 -
Case Reports in Otolaryngology 2017A 70-year-old female presented with a neck mass and sporadic dry cough, often leading to fits of coughing severe enough to cause vomiting. The patient reported that...
A 70-year-old female presented with a neck mass and sporadic dry cough, often leading to fits of coughing severe enough to cause vomiting. The patient reported that touching the mass triggered the cough. On examination, a 2.5 cm right-sided level two neck mass deep to the sternocleidomastoid was present. Palpation of the mass immediately triggered coughing. Cross-sectional imaging proposed vagal paraganglioma as the chief differential, which was confirmed following surgical excision. The patient reported complete resolution of her severe dry cough after surgery. Vagal paragangliomas are rare neuroendocrine tumors arising from the neural crest-derived paraganglionic tissue surrounding the vagus nerve, typically presenting as a neck mass associated with hoarseness or pulsatile tinnitus. To the best of our knowledge this is a unique description in the English literature. This case is presented to aid physicians should they encounter a neck mass associated with cough. Vagal paraganglioma, although rare, should be part of the differential in such a presentation.
PubMed: 28717527
DOI: 10.1155/2017/7603814 -
Acta Otorhinolaryngologica Italica :... Oct 2014The aim of this study was to describe our experience with benign parapharyngeal space tumours resected via a transcervical route without mandibulotomy and to investigate...
The aim of this study was to describe our experience with benign parapharyngeal space tumours resected via a transcervical route without mandibulotomy and to investigate associated postoperative sequelae and complications. The study investigated and analysed the retrospective charts of 44 patients who underwent surgery for benign parapharyngeal space tumours over a 10-year period. The diagnosis was reached in all patients with clinical and radiologic findings; preoperative fine-needle aspiration biopsy was not performed in any case. The preferred means of accessing the parapharyngeal space in all patients was a transcervical route. In 5 of these patients, transparotid extension was performed due to the position of the tumour. Tumours were classified radiologically as poststyloid in 27 cases and prestyloid in 17 cases. The final histopathologic diagnosis was vagal paraganglioma in 16 cases, pleomorphic adenoma in 13 cases, schwannoma in 10 cases and comparatively rarer tumours in the remaining 5 cases. In three patients, cranial nerve paralysis was observed during preoperative evaluation. Permanent cranial nerve paralysis occurred in 19 cases (43.2%) in the postoperative period, the majority of which were neurogenic tumours such as vagal paraganglioma (n = 16) and schwannoma (n = 2), and one case of non-neurogenic parapharyngeal tumour. The median duration of follow-up was 61 ± 33 months. There was no local recurrence in any patient during the follow-up period. A transcervical approach should be the first choice for excision of parapharyngeal space tumours, except for recurrent or malignant tumours, considering its advantages of providing direct access to the neoplasm, adequate control of neurovascular structures from the neck and optimal aesthetic outcomes due to preservation of mandibular continuity with minimal morbidity and hospitalisation time.
Topics: Adenoma, Pleomorphic; Adult; Aged; Female; Follow-Up Studies; Head and Neck Neoplasms; Humans; Male; Middle Aged; Neurilemmoma; Paraganglioma; Pharynx; Retrospective Studies; Surgical Procedures, Operative
PubMed: 25709146
DOI: No ID Found -
Polish Journal of Radiology 2016Hereditary head and neck paragangliomas (HNP) are very often associated with pheochromocytoma-paraganglioma syndromes, which are caused by mutations in genes encoding...
BACKGROUND
Hereditary head and neck paragangliomas (HNP) are very often associated with pheochromocytoma-paraganglioma syndromes, which are caused by mutations in genes encoding subunits of succinate dehydrogenase () complex. The aim of this study was to determine the frequency and location of HNP among SDHx carriers.
MATERIAL/METHODS
A total of 72 patients with mutations underwent computed tomography examinations of the head and neck. HNP were present in 44 (61.1%) out of 72 patients (31 , 11 , 2 ); 113 HNP were found; the most common were carotid paragangliomas (59) and vagal paragangliomas (27).
RESULTS
The HNP were statistically more frequent in carriers of mutations compared to carriers of mutations (72.1% 43.5%, p=0.033). Multiple tumors more often occurred in patients with mutations 26/31 (83.9%) than in patients with mutations 6/11 (54.5%) p=0.05. There was a significant difference in the prevalence of carotid paragangliomas between patients with and mutations (7/11 [63.6%] 30/31 [96.8%], respectively, p=0.004). Patients with mutations more often had carotid paragangliomas located on the left side than on the right side, as compared to mutations 25/31 (80.6%) 4/11 (36.4%), p=0.006.
CONCLUSIONS
mutations predispose to multifocal and bilateral HNP. Carotid and vagal paragangliomas occurred most often. Patients with mutations are characterized by higher frequency of HNP than patients with mutations, which is mainly driven by higher frequency of carotid body tumors in patients with mutations. No difference in the frequency of head and neck paragangliomas in other locations was found.
PubMed: 27867439
DOI: 10.12659/PJR.897490 -
International Cancer Conference Journal Jan 2022Carotid body tumor involving the succinate dehydrogenase subunit B (SDHB) variant reportedly had a higher frequency of metastasis than other variants of succinate...
UNLABELLED
Carotid body tumor involving the succinate dehydrogenase subunit B (SDHB) variant reportedly had a higher frequency of metastasis than other variants of succinate dehydrogenase. However, the correlation between genotype and phenotype among patients with carotid body tumor with gene variant remains unclear. Thus, we present a case of carotid body tumor with neck lymph metastasis caused by a novel variant, which resulted in long-term disease-free survival achieved after surgery. A 43-year-old man presented to our hospital with a 2-year history of a painless neck mass. Based on the radiographic findings, the patient was diagnosed with carotid body tumor with a possible Shamblin type III tumor. Another mass was detected and suspected to be a lymph node metastasis. The patient underwent resection of the tumor and lymph nodes. The common carotid artery, internal carotid artery, external carotid artery, internal jugular vein, vagal nerve, and hypoglossal nerve were resected with the tumor. Histopathological examination revealed a paraganglioma. The histological findings of the lymph nodes were similar to those of the carotid body tumor and were confirmed to be metastases of paraganglioma. To analyze the germline variant, a nonsense variant was detected in the gene at exon 2, c. 136C > T, p. Arg46*. During the follow-up 80 months after surgery, the patient exhibited no signs of recurrence, metastasis, or development of paragangliomas in other organs. This was the first case of carotid body tumor accompanied by neck metastasis caused by a germline nonsense variant at exon 2, c. 136C > T, p. Arg46*. Carotid body tumor with neck lymph metastasis caused by this nonsense variant could achieve long-term disease-free survival after surgery. Gene analysis, including variant, should be performed to predict the prognosis and future risk of metastasis. Genetic testing of may give a crucial information for the treatment and follow-up strategies of carotid body tumor.
SUPPLEMENTARY INFORMATION
The online version contains supplementary material available at 10.1007/s13691-021-00522-x.
PubMed: 35127314
DOI: 10.1007/s13691-021-00522-x -
Head & Neck Jun 2019F-FDOPA PET/CT was proved to be a highly sensitive imaging method for detecting head and neck paraganglioma (HNPGL). The primary aim of the study was to evaluate the...
BACKGROUND
F-FDOPA PET/CT was proved to be a highly sensitive imaging method for detecting head and neck paraganglioma (HNPGL). The primary aim of the study was to evaluate the relationship between tumor characteristics and the SDHx-mutational status in a large series of patients with HNPGL evaluated by F-FDOPA PET/CT.
METHODS
A total of 104 patients with HNPGL (65 sporadic/39 SDHx-mutated) were included.
RESULTS
In comparison to SDHB/SDC/SDHx-negative cases, patients with SDHD were younger at diagnosis and had a higher rate of multifocal, vagal, and carotid paraganglioma. In patients with SDHD, vagal paraganglia represented the primary site of tumor origin. Multicentric involvement of the vagus nerve alone or in association with other locations was found to be a typical feature of SDHD cases compared to other cases (odds ratio = 59.4).
CONCLUSION
The present study shows that tumor multifocality within the vagus nerve is a phenotypic marker of SDHD mutation. This information is essential in the choice of the therapeutic strategy.
Topics: Age Factors; Cranial Nerve Neoplasms; Dihydroxyphenylalanine; Female; Fluorine Radioisotopes; Head and Neck Neoplasms; Heterozygote; Humans; Male; Middle Aged; Mutation; Neoplasms, Multiple Primary; Paraganglioma, Extra-Adrenal; Phenotype; Positron Emission Tomography Computed Tomography; Retrospective Studies; Succinate Dehydrogenase; Vagus Nerve; Vagus Nerve Diseases
PubMed: 30584686
DOI: 10.1002/hed.25604 -
Cureus Aug 2022Head and neck paragangliomas (PGLs) most commonly derive from the carotid body, jugulotympanic, vagal, and laryngeal paraganglia. Thyroid PGLs originate in the inferior...
Thyroid Paraganglioma With Medullary Carcinoma: A Unique Combination in a Patient in Association With Multiple Endocrine Neoplasia Type 2B Syndrome With Prolonged Survival.
Head and neck paragangliomas (PGLs) most commonly derive from the carotid body, jugulotympanic, vagal, and laryngeal paraganglia. Thyroid PGLs originate in the inferior laryngeal paraganglion, which may lie inside the thyroid parenchyma. Intrathyroid PGLs are rare with approximately 75 cases reported to date, mostly as solitary lesions. The coexistence of thyroid PGL with medullary thyroid carcinoma (MTC) has not been reported. Here, we report a unique case of intrathyroid PGL concomitant with MTC in the context of multiple endocrine neoplasia type 2B syndrome. Interestingly, the patient showed a prolonged survival with good clinical response to tyrosine kinase inhibitors, despite her advanced metastatic MTC. We discuss the challenges in pathology, differential diagnosis, and genetic background for the development of these thyroid lesions.
PubMed: 36176816
DOI: 10.7759/cureus.28423 -
The American Journal of Case Reports Oct 2023BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived...
BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived tumors, also called non-chromaffin, are located almost exclusively in the neck and skull base and are usually non-secretory and inactive. A case of malignant glomus jugulare with a metastatic cervical lymph node is described here. CASE REPORT A 24-year-old woman was referred to an otolaryngology clinic for concern of voice change for 1 month, which was associated with right progressive hearing loss, pulsating tinnitus, and right facial weakness. A clinical examination revealed a reddish mass in the right ear behind an intact tympanic membrane with right facial weakness of House-Brackmann grade VI. A bedside flexible nasopharyngoscopy revealed an immobile right vocal fold. A computed tomography scan of the brain revealed a destructive lesion within the right jugular foramen. The patient underwent embolization followed by glomus tumor resection via infra-temporal fossa with Fisch type A approach. Pathology revealed that the tumor was an infiltrative epithelioid tumor with a spindle and nesting pattern separated by fibrovascular stroma. The submitted lateral neck lymph node revealed a metastatic tumor. CONCLUSIONS Glomus jugulare tumors are uncommon paragangliomas, and malignant behavior with metastasis is extremely rare. Metastatic tumors are often associated with facial and vagal nerves palsy. There are no histological features that distinguish malignant glomus jugulare tumors. Malignant neoplasms are characterized by the presence of metastases. Tumors of the glomus jugulare that are malignant are treated with surgery, radiotherapy, or both. However, our search of the literature revealed no clear guidelines, given the scarcity of cases. Moreover, the presence of metastasis increases the risk of death.
Topics: Female; Humans; Young Adult; Adult; Glomus Jugulare Tumor; Glomus Tumor; Paraganglioma; Facial Paralysis; Skull Base; Neoplasms, Second Primary; Sarcoma
PubMed: 37814445
DOI: 10.12659/AJCR.940138 -
Case Reports in Veterinary Medicine 2020Pheochromocytomas are catecholamine-secreting tumors that are composed of neuroectoderm-derived chromaffin cells. An 8-year-old miniature dachshund with abdominal...
Pheochromocytomas are catecholamine-secreting tumors that are composed of neuroectoderm-derived chromaffin cells. An 8-year-old miniature dachshund with abdominal distension was diagnosed with a neuroendocrine tumor with invasion from the caudal vena cava to the right ventricular cavity. The dog died due to hypotensive shock from the vagal reflex, and on autopsy, an extra-adrenal pheochromocytoma (paraganglioma) was diagnosed in the caudal abdomen. At autopsy, the tumor plug of the caudal vena cava was confirmed. To the best of our knowledge, this is the first case report that echo-captured the extension of pheochromocytoma in the right ventricle and shows it in a figure and video file.
PubMed: 32095312
DOI: 10.1155/2020/5382687